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目的 探讨伴有皮层下梗死和白质脑病 (Cerebralautonomicdominantarteriopathywithsubcorti calinfarctsandleukoencephalopathy ,CADASIL)的常染色体显性遣传性脑动脉病的临床特点和诊断方法。 方法 对 1例CADASIL患者的临床表现、影像学 (MRI、CT)特点及皮肤活检等方面进行了探讨。结果 患者临床表现为反复发作的缺血性脑卒中、记忆力减退、假性球麻痹、MRI、CT见皮质下梗死和白质脑病的改变 ,皮肤活检显示小动脉含糖原颗粒 ,管腔狭窄 ,血管内皮下黑色嗜锇颗粒。结论 通过本病的临床特点、影像学和皮肤活检 ,可在生前进行诊断。
Objective To investigate the clinical features and diagnostic methods of autosomal-dominant degenerative cerebral artery disease with subcortical infarction and cerebral encephalopathy (Cerebralactonomicdominantartiopiopathywithsubcorti calinfarcts and leukemiaencephalopathy, CADASIL). Methods One case of CADASIL patients with clinical manifestations, imaging (MRI, CT) features and skin biopsy were discussed. Results The clinical manifestations were recurrent ischemic stroke, memory loss, pseudobulbar palsy, MRI and CT findings of subcortical infarction and leukoencephalopathy. Skin biopsy showed that the arterioles containing glycogen granules, stenosis and blood vessels Under the skin black osmiophilic particles. Conclusion The clinical features of this disease, imaging and skin biopsy can be diagnosed before birth.